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We report the most severe case to date of an intrauterine growth\u2010restricted term male who presented with severe acidosis and a high anion gap soon after birth. The manifestation was fatal that led to death within 36\u2009hours of life. The diagnosis was made postnatally by Whole Genome Sequencing (WGS). We report a rapid and fatal event of HIBCN in a neonate and review of the literature.<\/jats:p>","DOI":"10.1155\/2024\/8099373","type":"journal-article","created":{"date-parts":[[2024,6,30]],"date-time":"2024-06-30T01:19:52Z","timestamp":1719710392000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Fatal Case of 3\u2010Hydroxyisobutyryl\u2010CoA Hydrolase Deficiency in a Term Infant with Severe High Anion Gap Acidosis and Review of the Literature"],"prefix":"10.1155","volume":"2024","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7467-5874","authenticated-orcid":false,"given":"Surasak","family":"Puvabanditsin","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-5051-9722","authenticated-orcid":false,"given":"Ian","family":"Lee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-1306-9692","authenticated-orcid":false,"given":"Natasha","family":"Cordero","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keisha","family":"Target","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-5995-9123","authenticated-orcid":false,"given":"Su Young","family":"Park","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3075-4498","authenticated-orcid":false,"given":"Rajeev","family":"Mehta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,6,29]]},"reference":[{"key":"e_1_2_10_1_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41439-023-00251-y"},{"key":"e_1_2_10_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/ccr3.4528"},{"key":"e_1_2_10_3_2","doi-asserted-by":"publisher","DOI":"10.5582\/irdr.2019.01014"},{"key":"e_1_2_10_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2015.05.008"},{"key":"e_1_2_10_5_2","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-188"},{"key":"e_1_2_10_6_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.70.4.532"},{"key":"e_1_2_10_7_2","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1002\/ccr3.1998","article-title":"Beta-hydroxyisobutyryl coenzyme A deacylase deficiency: a defect in valine metabolism associated with physical malformations child with novel HIBCH compound heterozygous mutations","volume":"7","author":"Brown G. 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